Lecture 11- Immune system induced diseases 1 Flashcards
What is primary immunodeficiency?
It is a defect in one or more components of the immune system (either innate or adaptive systems). They typically do not show until they are needed.
What is the major clinical sign that indicates PID?
Constant infections and abnormal responses to microbes.
What are the three types of primary immunodeficiency?
Antibody deficiencies, t-lymphocyte deficiencies and innate deficiencies
What is Common Variable Immunodeficiency (CVID)?
It is a B-cell malfunction in which the cells fail to differentiate into the plasma cells. As a result, no antigens are produced at all.
What is the treatment for CVID?
Immunoglobulin replacement therapy (antibody transplants) and antibiotics.
what is specific antibody deficiency?
It is a deficiency in a certain antibody, IgG 2. This results in no protection from polysaccharide antigens.
What is the major pathological outcome from having specific antibody deficiency?
They get infections from the polysaccharide antigen, streptococcus pneumoniae. This results in pneumonia.
what is severe combined immunodeficiency?
It is very rare, but it basically involves a lack of B and T-cells in the body. This causes severe susceptability to infections and requires strong isolation from the environment (bubble boy).
What is IPEX syndrome?
It is a lack of the Treg cells in the immune system. This results in an absence of small bowel mucosa and inflammatory infiltrate in many organs.
What is IRAK-4 deficiency?
It is a deficiency in the interleukin-1 receptor associated kinase 4. This results in impaired T-cell activated.
